Difference between revisions of "Hirschsprung's disease"

(Clinical Features)
 
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***Distended abdomen, [[Nausea and vomiting (peds)|bilious vomiting]], enterocolitis
 
***Distended abdomen, [[Nausea and vomiting (peds)|bilious vomiting]], enterocolitis
 
*Enterocolitis
 
*Enterocolitis
**[[Diarrhea (peds)|Diarrhea]], rectal bleeding, abdominal distention, [[fever]]
+
**[[Diarrhea (peds)|Diarrhea]], [[GI Bleeding (Peds)|rectal bleeding]], abdominal distention, [[fever]]
  
 
==Differential Diagnosis==
 
==Differential Diagnosis==

Latest revision as of 21:18, 29 September 2019

Background

  • Congenital aganglionosis of colon
  • Usually affects rectosigmoid area
    • Short segment affects rectum and small amount of distal colon
    • Long segment affects more of colon, proximal intestine
    • Rarely involves more proximal intestine
  • 30% associated with a syndrome
  • 1:5,000 live births

Clinical Features

Differential Diagnosis

Infant Constipation

Evaluation

  • KUB distended colon, empty rectum
    • Enterocolitis: distention, bowel wall thickening
    • Obstruction: air fluid levels
  • Barium/contrast enema will show narrowed colon and dilated intestine above
    • Sensitivity 70% and specificity of 83%
  • Diagnosis requires rectal biopsy (consult GI)

Management

  • Consult pediatric surgeon for operative management
  • ED management may include gastric and rectal decompression
  • Treat enterocolitis with broad spectrum antibiotics
  • Supportive fluid and electrolyte replacement

Disposition

  • Admit if symptoms of obstruction, enterocolitis

See Also

External Links

References